Objective To investigate the drug effect of children with Dent disease who received therapy of po-tassium citrate and thiazide diuretics and angiotensin -converting enzyme inhibitors(ACEI),and to provide the refe-rence for the clinical treatment.Methods Dent disease patient who were followed -up in Bayi Children′s Hospital Af-filiated to Beijing Military Region General Hospital during the period of July 2006 and March 201 4 were selected.The patients were administered a therapy of potassium citrate associated with thiazide diuretics and ACEI according to the level of proteinuria and calciuria and serum potassium.The underlying changes before and after the treatment were com-pared and analyzed.Results In 1 5 children with Dent disease,they were all male cases,onset age ranged from 3 months to 1 1 years old[(2.62 ±3.1 1 )years old],and the disease duration ranged from 0.50 to 9.50 years old [(2.81 ±2.34)years].The patients were followed up for 0.50 to 7.50 years[(3.61 ±2.62)years].There was a sig-nificantly statistical difference in calcium/creatinine and daily Ca -creat ratio in contrast to before treatment[(0.41 ± 0.1 9)mg/mg vs(0.26 ±0.1 2)mg/mg,t =2.603,P =0.021 ;(6.76 ±2.0)mg/kg vs (4.34 ±1 .97)mg/kg,t =5.265,P =0.000],there was no significantly statistical difference in 24 -hour urinary protein quantity in contrast to before treatment[(0.96 ±0.62)g/24 h vs (0.87 ±0.44)g/24 h,t =1 .01 6,P =0.327].One case with kidney stone and 5 cases with nephrocalcinosis had a negative result of renal ultrasound after treatment.Conclusions Treatment of potassium citrate combination with thiazide diuretics and ACEI can significantly decrease urinary calcium excretion, make a disappearance of kidney stone and nephrocalcinosi,and it may have a role in protecting renal function.Treat-ment of benazepril can not significantly decrease the proteinuria and has no substantial improvement in low molecular weight protein urine.%目的：探讨枸橼酸钾联合氢氯噻嗪及肾素血管紧张素转换酶抑制剂（ACEI）治疗 Dent 病患儿的药物疗效，为 Dent 病的药物治疗提供临床依据。方法选取2006年7月至2014年3月北京军区总医院附属八一儿童医院随访的 Dent 病患儿。根据尿蛋白、尿钙及血钾水平，予枸橼酸钾联合氢氯噻嗪及 ACEI 类药物治疗。比较分析其治疗前后各项指标的变化。结果15例患儿均为男性；发病年龄3个月～11岁[（2．62±3．11）岁]；病程0．50～9．50（2．81±2．34）年。共随访0．50～7．50年（3．61±2．26）年。治疗后，尿钙／肌酐水平与治疗前比较差异有统计学意义[（0．41±0．19）mg／mg 比（0．26±0．12）mg／mg，t ＝2．603，P ＝0．021]，24 h尿钙定量差异有统计学意义[（6．76±2．05）mg／kg 比（4．34±1．97）mg／kg，t ＝5．265，P ＝0．000]；24 h 尿蛋白定量差异无统计学意义[（0．96±0．62）g／24 h 比（0．87±0．44）g／24 h，t ＝1．016，P ＝0．327]。肾脏超声：原有1例肾结石患儿超声显示结石消失，原有5例存在肾脏钙化灶患儿治疗后肾脏钙化灶消失。结论枸橼酸钾联合氢氯噻嗪及 ACEI 类药物治疗可降低 Dent 患儿的尿钙水平，使肾脏钙化灶或结石消失，可能具有保护肾功能的作用。但贝那普利在降低尿蛋白水平上作用不明显，对低分子蛋白尿无明显改善。
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